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Transitions from Inhaled, Intravenous, Subcutaneous, or Oral Prostacyclins to Selexipag: Interim Data from the SPHERE Registry (SelexiPag: tHe UsErs dRug rEgistry)

Anna Hemnes

Harrison Farber

Nick H. Kim

Kelly Chin

Murali Chakinala

Kristin Highland

Carol Zhao

Michael Keating

Vallerie McLaughlin


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Conference: 2018 PHA International PH Conference & Scientific Sessions

Release Date: 06.28.2018

Presentation Type: Abstracts

File Download: Conference 2018_1029

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2018 International PH Conference and Scientific SeAbstract presented at the 2018 International PH Conference and Scientific Sessions in Orlando, Fla., June 28-July 1, 2018.


The selective oral IP prostacyclin receptor agonist selexipag is approved to delay disease progression and reduce the risk of hospitalization in pulmonary arterial hypertension (PAH) patients. SPHERE is a US registry of PAH patients designed to provide data on real-world selexipag use. Here we report data on patients transitioning from inhaled, parenteral, or oral prostacyclins to selexipag.


Enrollment began in November 2016 with a goal of enrolling 500 patients. Patients previously or newly initiated on selexipag are eligible, however previously initiated patients must have a documented titration regimen. Data are collected at routine clinic visits and patients are followed for up to 18 months. Transitioning patients were defined as those taking a prostacyclin for ≥30 days at the time of selexipag initiation or until ≤7 days before selexipag initiation.


This analysis (data cut-off October 2, 2017) reports data from 250 patients enrolled from 46 sites. The median time since PAH diagnosis was 4.2 years (range: 0.0–63.3). The median total duration of selexipag treatment was 11.6 months (range: 0.1–21.5). The median on-study duration was 3.1 months (range: 0.1–12.1). Sixty patients (24%) were transitioned to selexipag from prostacyclin and 190 (76%) were non-transitioned. At selexipag initiation, transitioning patients had a longer mean 6MWD (324.2 m [SD 175.4] vs. 312.0 m [SD 139.0]) than non-transitioned patients and a similar proportion were FC I/II (32% vs. 28%) and III/IV (58% vs 49%). At transition, 30 patients were on triple therapy consisting of an ERA, a PDE5i/sGC, and an inhaled (n=14), intravenous (n=10), oral (n=4), or subcutaneous (n=2) prostacyclin; and 19 were on dual therapy consisting of an ERA and a prostacyclin (n=11), or a PDE5i/sGC and a prostacyclin (n=8). Seven patients transitioned from inhaled (n=5) or oral (n=2) treprostinil monotherapy to selexipag monotherapy. Four patients were not taking a prostacyclin at the time of selexipag initiation but had discontinued their prostacyclin therapy for ≤7 days. Patients who transitioned from another prostacyclin to selexipag had a higher median maintenance dose of selexipag compared to non-transitioned patients (1400 µg BID [range: 200–1600] vs. 1200 µg BID [range: 100–1600]). The incidence and type of adverse events were similar between the two groups.


In this real-world dataset, patients who transitioned to oral selexipag were most commonly on triple therapy and had a higher median selexipag maintenance dose compared to non-transitioned patients. Tolerability was similar between transitioned and non-transitioned patients.